Familial Mediterranean fever (FMF) is the most common hereditary autoinflammatory disease. We aimed to investigate the oral health status and oral hygiene habits in children with FMF. In this cross-sectional study, 199 children with FMF, aged between 3-18 years, were included. Demographic findings and oral hygiene habits of children were questioned by face-to-face interview. Oral health status of patients was evaluated using decay-missing-filled index [DMFT (decay-missing-filled teeth), DMFS

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... cay-missing-filled teeth) for permanent; dmft, dmfs for primary teeth], the International Caries Detection and Assessment System (ICDAS-II) index, PUFA / pufa index [the presence of severely decayed teeth with visible pulpal involvement (P/p), ulceration caused by dislocated tooth fragments (U/u), fistula (F/f) and abscess (A/a)], gingival (GI) and plaque index (PI). In addition to these, occlusion, oral soft and hard tissues were examined. One-hundred-nine (54.8%) of children had at least one decayed permanent tooth and 81.2% of children had at least one decayed primary tooth. The mean DMFT was 1.91±2.45, DMFS was 3.1±4.49, dmft was 3.95±3.54, dmfs was 8.62±8.88, PI was 1.17±0.44, GI was 0.85±0.39. Aphthous mouth ulcer occurred in 19 (9.5%) patients. Recurrent aphthous mouth ulcers were more frequent among patients with one exon-ten and one exon-two mutations than patients with one exon-10 mutation, two exon-ten mutations, or two exon-2 mutations (61.1% vs. 47.9%, 26.1%, 20%, respectively p < 0.001). Tooth decay was more frequent among patients who had attacks in the last six months than those who did not have any attacks during the last six months (97.4% vs. 87.7%, p=0.017). Dental caries and periodontal disease, which are public health problems, were seen at a high percentage of children with FMF in our study.